1wt6

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[[Image:1wt6.gif|left|200px]]
 
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{{Structure
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==Coiled-Coil domain of DMPK==
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|PDB= 1wt6 |SIZE=350|CAPTION= <scene name='initialview01'>1wt6</scene>, resolution 1.60&Aring;
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<StructureSection load='1wt6' size='340' side='right'caption='[[1wt6]], [[Resolution|resolution]] 1.60&Aring;' scene=''>
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|SITE=
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== Structural highlights ==
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|LIGAND=
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<table><tr><td colspan='2'>[[1wt6]] is a 3 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1WT6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1WT6 FirstGlance]. <br>
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|ACTIVITY=
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.6&#8491;</td></tr>
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|GENE=
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1wt6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1wt6 OCA], [https://pdbe.org/1wt6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1wt6 RCSB], [https://www.ebi.ac.uk/pdbsum/1wt6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1wt6 ProSAT]</span></td></tr>
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|DOMAIN=
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</table>
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|RELATEDENTRY=
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== Disease ==
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1wt6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1wt6 OCA], [http://www.ebi.ac.uk/pdbsum/1wt6 PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1wt6 RCSB]</span>
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[https://www.uniprot.org/uniprot/DMPK_HUMAN DMPK_HUMAN] Defects in DMPK are the cause of dystrophia myotonica type 1 (DM1) [MIM:[https://omim.org/entry/160900 160900]; also known as Steinert disease. A muscular disorder characterized by myotonia, muscle wasting in the distal extremities, cataract, hypogonadism, defective endocrine functions, male baldness and cardiac arrhythmias. Note=The causative mutation is a CTG expansion in the 3'-UTR of the DMPK gene. A length exceeding 50 CTG repeats is pathogenic, while normal individuals have 5 to 37 repeats. Intermediate alleles with 35-49 triplets are not disease-causing but show instability in intergenerational transmissions. Disease severity varies with the number of repeats: mildly affected persons have 50 to 150 repeats, patients with classic DM have 100 to 1,000 repeats, and those with congenital onset can have more than 2,000 repeats.<ref>PMID:1546326</ref> <ref>PMID:1310900</ref> <ref>PMID:1302022</ref> <ref>PMID:19514047</ref>
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}}
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== Function ==
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[https://www.uniprot.org/uniprot/DMPK_HUMAN DMPK_HUMAN] Non-receptor serine/threonine protein kinase which is necessary for the maintenance of skeletal muscle structure and function. May play a role in myocyte differentiation and survival by regulating the integrity of the nuclear envelope and the expression of muscle-specific genes. May also phosphorylate PPP1R12A and inhibit the myosin phosphatase activity to regulate myosin phosphorylation. Also critical to the modulation of cardiac contractility and to the maintenance of proper cardiac conduction activity probably through the regulation of cellular calcium homeostasis. Phosphorylates PLN, a regulator of calcium pumps and may regulate sarcoplasmic reticulum calcium uptake in myocytes. May also phosphorylate FXYD1/PLM which is able to induce chloride currents. May also play a role in synaptic plasticity.<ref>PMID:10913253</ref> <ref>PMID:10811636</ref> <ref>PMID:11287000</ref> <ref>PMID:15598648</ref> <ref>PMID:21457715</ref> <ref>PMID:21949239</ref>
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'''Coiled-Coil domain of DMPK'''
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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==Overview==
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<jmolCheckbox>
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The coiled-coil domain of dystrophia myotonica protein kinase (DMPK) has been cloned, overexpressed, purified and crystallized. Two crystal forms have been obtained that belong to space groups P3 and P2(1)2(1)2(1) and diffract to 2.4 and 1.6 A resolution, respectively. Experimental phases were obtained by MAD from an SeMet derivative. The location of selenium sites used molecular-replacement phases obtained from search models lacking sequence similarity with the coiled-coil under study. Both crystal forms contain three polypeptide chains in the asymmetric unit.
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/wt/1wt6_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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==About this Structure==
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<text>to colour the structure by Evolutionary Conservation</text>
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1WT6 is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1WT6 OCA].
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1wt6 ConSurf].
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==Reference==
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<div style="clear:both"></div>
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Crystallization and preliminary X-ray analysis of the coiled-coil domain of dystrophia myotonica kinase., Garcia P, Marino M, Mayans O, Acta Crystallogr D Biol Crystallogr. 2004 Dec;60(Pt 12 Pt 2):2336-9. Epub, 2004 Nov 26. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/15583383 15583383]
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Garcia, P.]]
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[[Category: Garcia P]]
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[[Category: Marino, M.]]
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[[Category: Marino M]]
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[[Category: Mayans, O.]]
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[[Category: Mayans O]]
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[[Category: coiled-coil]]
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[[Category: dmpk]]
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[[Category: kinase activation]]
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[[Category: molecular replacement]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Mar 31 00:40:41 2008''
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Current revision

Coiled-Coil domain of DMPK

PDB ID 1wt6

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